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GlossaryIn your cancer journey you will come across many terms which need explaining. We cannot offer a complete list of explanations here but if you spot any that should be added please let us know.A B C D E F G H I J K L M N O P Q R S T U V W X Y Z Adjuvant treatment - usually treatment as a follow-up to surgery designed to remove any microscopic traces of tumour which may be left behind. Alveolar soft part sarcoma - a sarcoma of indeterminate origin though probably related to rhabdomyosarcoma. Angiosarcoma - sarcoma of the blood vessels. This is a sarcoma where known causes include chemical exposure and radiotherapy for (typically) breast cancer. AP23573 - a new drug developed by Ariad. Now known as ridaforolimus. Has had successful early phase trials in USA with some excellent indications for advanced soft tissue sarcoma. A large-scale Phase 3 trial in Europe and North America has just been completed. Askin's Tumour - a member of the Ewings family of tumours. It is a tumour of the periosteum, the membrane which surrounds bones. Bad Nauheim Declaration - an international declaration by GIST advocacy groups demanding common standards of treatment for all patients using consensus guidelines which are evidence-based. The Declaration. Chondrosarcoma - sarcoma usually affecting bone but is also known in soft tissue. It has its origin in cartilage. Many variants which are revealed by pathology. Chordoma - a very rare bone cancer in the spine. Cisplatin - a platinum based chemotherapy which is used in treating osteosarcoma. Clear cell sarcoma - this can sometimes be mistaken for metastatic melanoma so careful diagnosis is important. Can affect tendons. CNS - cancer nurse specialist. Desmoid tumour - fibromatosis, a rare fibrous tumour which can be locally aggressive but does not metastasise. DFSP - dermatofibrosarcoma protuberans. A rare fibrous sarcoma usually in the skin layers. Rarely metastasises to other areas though it can spread locally. Responds to Glivec. Docetaxel - a chemotherapy drug being tested in combination with gemcitabine to treat soft tissue sarcomas. Doxorubicin - chemotherapy drug used as 'first-line' treatment in soft tissue sarcoma and often included in combination therapy for bone sarcomas. Endometrial Stromal Sarcoma (ESS) - a uterine sarcoma arising in the endometrium. Usually diagnosed and treated within the gynaecology speciality. It accounts for less than 0.5% of uterine cancers. Patients with positive tumours who are taking HRT should take advice. If their tumours metastasise they may respond to a new class of drugs known as aromatase inhibitors. Endo-prostheses - artificial bones used in limb salvage surgery for bone sarcomas. Epithelioid sarcoma - a difficult sarcoma to diagnose as it often mimics other benign and malignant conditions. Often found on the hand. Unlike most sarcomas is known to spread through the lymph system. ET743 - see Yondelis/trabectedin. Etoposide - a chemotherapy used in combination in bone sarcoma treatment. Ewing's sarcoma - a bone sarcoma most commonly found in teenagers. It is named after the doctor who first identified it. There are rare instances of Ewing's tumours in soft tissue. Gemcitabine - or Gemzar (Eli Lilly) - a chemotherapy drug which has shown some promise in early trials as a treatment in combination with docetaxel. GIST - gastrointestinal stromal tumour - a soft tissue sarcoma of the stomach and gut. Uniquely it responds well to Glivec which can give long periods of remission for patients with advanced/metastatic disease. A second-line therapy, Sutnet (sunitinib) is also licensed but is not yet approved for NHS use by NICE. Other new drugs are also showing promise in clinical trials. Glivec - imatinib originally STI571 (Novartis) - new generation therapy for treating GIST. Taken as a daily tablet it has revolutionised the treatment of advanced and metastatic GIST. Approved for use in NHS at 400mg but not at higher doses. Grade - the grade of a tumour is defined by the pathologist and is based on a measurement of how active the tumour is. A high grade cancer is more likely to spread. Hemangioendothelioma - a rare blood vessel tumour which often occurs in the skin, liver, and spleen. Ifosfamide - chemotherapy drug used as either first or second-line in soft tissue sarcoma and as one of a cocktail of drugs with bone sarcoma. Imatinib - see Glivec Kaposi's sarcoma - a sarcoma of surface tissues which appears in people of Mediterranean origin. It is also associated with reduced immune systems and a version of Kaposi's sarcoma can affect HIV positive patients. It is often treated similarly to a melanoma. Leiomyosarcoma - sarcoma of smooth muscle tissue. Smooth muscle is the muscle you cannot control. One of the commoner soft tissue sarcomas. LMS can occur as a uterine cancer and is then usually only found after surgery. Liposarcoma - sarcoma of fatty tissue. One of the commoner soft tissue sarcomas. MDT - multi-disciplinary team - the medical team responsible for cancer treatment. An MDT will include all the major specialities, including pathology, nursing and other healthcare staff. Metastasis (plural=metastases) - occurrence of cancer at a site distant from the first (or primary) tumour. In sarcoma this occurs most frequently in the lungs, in GIST it is usually in the liver. MFH - malignant fibrous histiocytoma - a sarcoma which can affect soft tissue or, more rarely, bone. The commonest sarcoma diagnosis. Mixed Mullerian Tumour - also known as carcinosarcoma. This is a gyanecological cancer with characteristics of both a sarcoma and a carcinoma. MPNST - malignant peripheral nerve sheath tumour. A rare soft tissue sarcoma also known as malignant schwannoma and neurogenic sarcoma. A known cause is inherited neurofibromatosis type 1 (NF1). Neo-adjuvant - treatment prior to surgery designed to improve the prospects of a successful operation. Often chemotherapy but sometimes radiotherapy. Oncologist - doctor specialising in cancer. Oncologists may be surgical (surgeons), clinical (experts in radiotherapy) or medical (experts in drug treatments). Osteosarcoma - the commonest of the bone sarcomas. It can occur at any age but is most often diagnosed in teenagers and young adults. In the more elderly it can be associated with Paget's disease. Pathologist - the laboratory expert behind the front-line clinical team. PNET - primitive neuroectodermal tumor - a bone sarcoma related to Ewings sarcoma. Radiologist - a clinical expert in use of imaging and the diagnostic interpretation of images. May also be familiar with other diagnostic techniques. Rhabdomyosarcoma - sarcoma of striated, or voluntary, muscle. The commonest soft tissue sarcoma in children. There are three sub-types - embryonal (usually in children), alveolar (usually in younger adults) and pleomorphic (most common in more elderly people). RCT - randomised controlled trial - usually a Phase 3 clinical study comparing a standard and a new therapy. Staging - a step in the clinical process when the MDT has all the results from tests, and possibly from surgery, and can decide by comparison with a standard description where in the disease pathway a patient is. This helps determine treatment and monitoring procedures. Sutent - sunitinib malate originally SU011248 (Pfizer). A new generation therapy for GIST patients who fail Glivec. Licensed in summer 2006 after successful trials in 2004/5. Now approved for use in NHS. Synovial sarcoma - associated with synovial tissue around joints. Thoracotomy - lung surgery. In some instances metastatic sarcoma on the lungs can be surgically removed. Trabectedin - see Yondelis. ULMS - uterime leiomyosarcoma. Uterine fibroids are usually leiomyomas, a benign smooth muscle tumour. They can develop into malignant sarcomas. ULMS accounts for about 2% of uterine cancers and is usually treated within the gynae cancer speciality. We recommend that tumours are tested for oestrogen and progesterone receptor status. Patients with positive tumours who are taking HRT should take advice. If their tumours metastasise they may respond to a new class of drugs known as aromatase inhibitors. Yondelis - (generic name = trabectedin though it has also been called ectneiascidin)(originally ET743 ). Developed by Spanish marine bio-technology company Pharmamar. Derived from Caribbean sea squirt. Licensed for use as second-line in soft tissue sarcoma following doxorubicin/ifosfamied. Approved by NICE in late 2009. Clinical evidence supports its use in leiomyosarcoma, myxoid liposarcoma and synovial sarcoma. Other sub-types will follow.
Page reviewed December 2009 |